Cells require thousands of unique proteins to be in the right place, at time, amounts and with modifications. They do this through several processes collectively known as protein homeostasis. TORC1 is a principal regulator homeostasis, coordinating synthesis degradation. The proteasome, composed core particle one or two regulatory particles, degrades unwanted protein. Diverse stresses cause hom...

Cardiac amyloidosis (CA) is characterized by progressive pathological deposition of amyloid fibrils in the myocardial interstitium, resulting ultimately restrictive cardiomyopathy. The accumulating heart are most commonly misfolded immunoglobulin light chains (in case AL amyloidosis) or transthyretin (ATTR), latter being either wild-type variant conformation.

Bioassays that involve the use of laboratory animals are often considered to be the ‘gold standard’ for the detection and titration of certain pathogens. This holds particularly true for prions,1,2 which are the proteinaceous infectious particles that cause fatal neurodegenerative diseases in animals (e.g. scrapie, bovine spongiform encephalopathy [BSE]) and humans (e.g. sporadic or variant Cre...

Protein misfolding and aggregation underlie several neurodegenerative proteinopathies including amyotrophic lateral sclerosis (ALS). Superoxide dismutase 1 (SOD1) was the first gene found to be associated with familial ALS. Overexpression of human mutant or wild type SOD1 in transgenic mouse models induces motor neuron (MN) degeneration and an ALS-like phenotype. SOD1 mutations, leading to the ...